Encephalocraniocutaneous lipomatosis is a congenital neurocutaneous syndrome with epibulbar choristomas and connective tissue nevi of the eyelids as common ophthalmic manifestations. Systemic manifestations occur ipsilateral to the ocular lesions and include lipomas of the cranium and central nervous system, alopecia of the scalp, and abnormalities of the central nervous system. We treated a child with encephalocraniocutaneous lipomatosis who required removal of an epibulbar choristoma. Pathologic evaluation of the epibulbar choristoma in our patient showed the presence of ectopic lacrimal gland tissue and cartilage. Encephalocraniocutaneous lipomatosis should be considered, together with Goldenhar's syndrome and sebaceous nevus syndrome, in the differential diagnosis of conditions associated with epibulbar choristomas.